【摘要】 目的探讨纤维素性纵隔炎的临床特征及预后,探讨不同检查在诊断与评估中的价值,并总结纤维素性纵隔炎诊断、评估流程。方法回顾性分析2015年1月至2020年9月间就诊于中日友好医院的28例纤维素性纵隔炎患者的临床资料,总结其临床表现、影像学特点、肺功能表现、气管镜下特征、超声心动图表现、肺通气灌注SPECT显像表现,以及右心导管检查结果和预后。结果患者中男8例,女20例,平均年龄69.9岁。主要临床症状有咳嗽(77.6%)、咳痰(57.1%)、喘息或喘憋(42.9%)、呼吸困难(39.3%),均表现为慢性病程。67.9%考虑既往结核或目前存在结核。影像学表现为纵隔内脂肪密度消失,纵隔不定型软组织影包绕气道及肺血管,多伴淋巴结肿大及钙化,多发支气管、肺血管受压狭窄。肺功能多表现为阻塞性通气功能障碍伴弥散量减低。支气管镜下表现为支气管黏膜色素沉着,支气管扭曲变形或多发狭窄,甚至闭塞,支气管黏膜水肿或充血。超声心动及右心导管检查示肺动脉高压、舒张性心功能不全是纤维素性纵隔炎常见的并发症。肺通气灌注SPECT显像示87.5%的患者存在血流灌注受损,37.5%的患者存在通气灌注受损。部分患者经抗感染、对症治疗后症状减轻,但纵隔纤维化不可逆,抗结核及糖皮质激素治疗效果有限。结论临床考虑纤维性纵隔炎的患者应行胸部增强CT明确诊断;完善肺功能、支气管镜、超声心动图检查,以评估该病是否累及气道、肺血管、心包,及其功能受累程度。重视对患者肺动脉高压、舒张性心功能不全的评估。更多还原

【Abstract】 Objective To investigate the clinical features and prognosis of fibrinous mediastinum and evaluate the value of different examinations in diagnosis and evaluation. Methods Twenty-eight patients with mediastinal fibrosis diagnosed between January 2015 and September 2020 in China-Japan Friendship Hospital were studied retrospectively.The Clinical manifestations, radiological characteristics, endoscopic features, echocardiography, V/Q SPECT, cardiac catheterization, treatment and prognosis were analyzed. Results The main clinical symptoms were cough(77.6%),expectoration(57.1%), wheezing or suffocating(42.9%), dyspnea(39.3%). There were 67.9% of the cases who were considered previous or present tuberculosis. Imaging findings showed that the fat density in the mediastinum disappeared,the irregular soft tissue of the mediastinum surrounded the airway and pulmonary vessels, and many lymph nodes enlarged and calcified, and multiple bronchus and pulmonary vessels were compressed and narrowed. Pulmonary function was mainly manifested as obstructive ventilate dysfunction and decreased diffusion volume. Under bronchoscopy, the bronchial mucosa showed pigmentation, bronchial distortion or multiple stenosis, even occlusion, and bronchial mucosa edema or congestion. Echocardiography and catheterization of the right heart showed that pulmonary hypertension and diastolic cardiac dysfunction were common complications of fibrillary mediastinum. Pulmonary ventilation perfusion imaging showed impaired blood perfusion in 87.5% of patients and impaired ventilation perfusion in37.5% of patients. The symptoms of some patients alleviated after anti-infective and symptomatic treatment, but the mediastinal fibrosis was irreversible, and the efficacy of anti-tuberculosis and glucocorticoid therapy was limited.Conclusions For patients with clinical consideration of fibrous mediastinum, chest enhanced CT should be performed for clear diagnosis. Relevant examinations, such as pulmonary function, endoscopic, echocardiography, should be conducted to evaluate whether the disease involves airway, pulmonary vessels, pericardium, superior vena cava and esophagus, as well as the degree of functional involvement. Attention should be paid to the evaluation of patients with pulmonary hypertension and diastolic cardiac insufficiency.更多还原